21 results
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... Features: alveolitis, ear ... myelodysplastic syndrome ... myeloid cells NEJM ... Autoinflammatory #Somatic #rheumatology
Sarcoidosis - Diagnosis and Management Summary
Epidemiology
1) High incidence in Scandinavian countries (11-24 cases per 100,000 individuals
Diagnosis and Management ... : (distinct symptoms ... ) • Löfgren syndrome ... Sarcoidosis #Diagnosis #Management ... #Signs #Symptoms
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
Dehydration • Symptoms ... changes, stroke symptoms ... tumor burden • Symptoms ... TLS #diagnosis #management ... #hematology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
meningitis), MCC CNS symptoms ... Giant retinal tears ... #diagnosis #management ... #signs #symptoms ... #rheumatology #
Susac Syndrome - Clinical Triad
 - Central nervous system dysfunction
 - Branch retinal artery occlusion
 -
Susac Syndrome - ... disease flares - 2-year ... David #Susac #Syndrome ... Triad #Diagnosis #Management ... #Rheumatology
Aplastic Anemia - Overview

Who?
• M = F
• 3 peaks:	1) Childhood	2) 15-25 years	3) >60 years

What?
• Aplastic anemia
Constitutional Syndromes ... Constitutional Syndromes ... • Improve the symptoms ... Anemia #oncology #hematology ... #diagnosis #management
Cryofibrinogenemia Summary

Cryofibrinogenemia Epidemiology:
 • 40-70 years with a modest female predominance

Cryofibrinogenemia:
 • The precipitation of a
of 37°C to the near-freezing ... Cryofibrinogenemia Symptoms ... Antiphospholipid syndrome ... Cryofibrinogenemia #diagnosis #rheumatology ... #hematology
Differentiation Syndrome in APML
Epidemiology:
 • Incidence: common in APL (2-48% depending on the study)
 • Triggers:
Differentiation Syndrome ... Diagnosis: >3 symptoms ... Differentiation #Syndrome ... APML #diagnosis #management ... #hematology #oncology
Giant cell arteritis (GCA)

Giant cell arteritis (GCA) definition: Most common systemic inflammatory vasculitis in older adults
in literature (NEJM ... cases (Cornelia NEJM ... them, but urgent rheumatology ... Temporal #Signs #Symptoms ... #Diagnosis #Management
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... HLH signs and symptoms ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology